Pediatric Heart Disorders: Expert Q&A

Chicagoland Children's Health Alliance is one of the largest, most comprehensive congenital heart programs in the region. Today on At the Forefront Live, we'll talk with experts as they discuss advancements in heart research and pediatric heart disorders, like aortic aneurysms, and the unique challenges to improve diagnoses, treatment, and outcomes. CCHA reflects a collaboration among University of Chicago Medicine Comer Children's Hospital, Advocate Children's Hospital, and Pediatrics at NorthShore University Health System. Our experts, Dr. Michael Earing and Dr. Luca Vricella will answer your questions coming up right now on At the Forefront Live.

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And we want to remind viewers that today's program is not designed to take the place of a visit with your physician. We're going to start off with having each of our physicians introducing themselves. And Dr. Earring, I'm going to start with you because you're right next to me. I know we argued a little bit about that beforehand, but I figured I'd throw a surprise to you.

[INAUDIBLE] I'm really excited to be here. My name is Dr. Michael Earing. I'm the chief of pediatric cardiology for the entire alliance. That's the University of Chicago Comers Children Program, Advocates Children Program, and then the Pediatrics at NorthShore. I'm also an adult-trained cardiologists, and I run the adult congenital heart program for the entire system as well, along with the Marfan program. So I'm really excited to be here, Tim.

Thank you. And when I say we argued, I'm teasing about that. It was just a little bit of a back and forth. But Dr. Vricella also joins us, and let's have you introduce yourself and tell us a little bit about what you do.

Sure, Tim. Thank you. First of all, I would like to welcome everybody to this live Q&A. My name is Luca Vricella. I'm a congenital cardiac surgeon, and I'm director of pediatric cardiovascular surgery across the entire alliance.

I've been doing this for 18 years, after 11 years of training. And together with my surgical colleagues, I actually specialize in the surgical treatment of all aspects of congenital heart disease. So we treat children from neonates, and we treat adults who are born with congenital heart disease and eventually need some surgical treatment. And so we really have a very large, broad spectrum of patients that go from, again, birth to adults.

And I know we're primarily focusing on pediatrics today, but I do want to talk a little bit about the adult side of things later on in the program. Dr. Earing, we're going to start with you with our questions. I do want to remind our viewers, though, as you're watching, you can type questions into the comments section. We'll try to get to as many as possible over the next half hour.

Let's start with talking a little bit about the Chicagoland Children's Health Alliance, because I think that's a fairly unique thing. And we were chatting before the program started. And bringing large health care institutions like this together is a pretty significant thing to have happen. And it's really a great thing for kids.

Yeah. No, it's great, Tim. For the first time with the alliance, you have the strength and the power and the energy of three major medical groups and medical centers in Chicago coming together. So you have the strength of Chicago Comer Children's Hospital. You have the strength of the Advocate Children's Hospital and then the pediatric subspecialty group at NorthShore.

Think about the power of all those physicians coming together and working together with the same quality, the same standards throughout the system, but more importantly, being able to reach all the patients in Chicago and the Chicagoland surrounding areas. This is really, really powerful. And it takes the best of all those systems and brings it together, and that is really one of the reasons why I came here, Tim, to help lead this with Dr. Vricella and the other physicians because this is exciting.

This has never been done in Chicago or the surrounding area. This opportunity is huge for the families and the patients in this area. Very exciting.

And when you talk about the area, this is a pretty broad area. We're not just talking about Chicago but northwest Indiana, Wisconsin, I would imagine, all throughout the state of Illinois. So it's a lot of kids that can really benefit from this.

We have over 30 different outreach sites. We have 50 physicians over another 50 nurse practitioners throughout the system, and we're able to expand all the way from Wisconsin into northern Indiana. We're out in the western part of the state, and our goal is really to have regional care.

We want to have care closer to home. We want to bring that care to all the families in your backyard. And regardless if it's a fetus, adult congenital patient, electrical problems of the heart, all those things locally. And only those surgeries and maybe those cath-based procedures would need to be done at one of the bigger centers.

And that's the goal, and that is such a powerful strength that we have with this alliance. And it's just an incredible opportunity for the patients and their families in the community. More importantly, we all communicate, working together, the hospital systems and medical records-- just really, really powerful.

And Dr. Vricella, it's great to have you here in Chicago. I know you're relatively new to the area. You've been here for a couple of years, I think, you were saying.

Yes, two years so far.

So talk to us a little bit about your background. You join us from Johns Hopkins, is that right?

Yeah. I've been at Johns Hopkins for 15 years. And this is, as you know, one of the most acclaimed institutions in the world. And what attracted me to come to Chicago was just the ability to do something even bigger.

So across this alliance, I think we can really accomplish so much and not only go-- go beyond the Chicagoland. So I think together we can go further, and we're already building on something that's incredibly strong. But we can do so much more.

So I was recruited with the idea of just potentiating the heart failure aspects of things, bring some other lines of services to what is already a very established, very prominent heart institute.

So let's talk about the congenital heart disease program, surgical side of it. And what differentiates what you do here, Dr. Vricella. And if you can, just in broad terms just explain congenital heart disease in general as well, please.

Sure. So congenital heart disease is the most frequent form of disease you're born with. I mean, so we're looking at just under 1% of all live births that have heart disease. And so it's the most common form of serious abnormality that children are born with.

And out of this, let's say, 1% of patients, live births with heart disease, you have three-quarters that have moderate or mild heart disease that will not need intervention in the first year of life. But you do have a quarter that actually will need intervention and the first year of life in the neonatal period or in early infancy because of the severity of this structural heart disease.

And this also has an impact on how long these patients will actually live. So if you have serious heart disease, your chances of being alive at age 18 are about 70%. This really underscores how serious congenital heart disease is and the impact that it has on the lives of children that are born with it. So it is really actually something that, as a society, has a huge impact.

Well, we do have our first question from a viewer-- and Dr. Vricella, since it's what you've just been speaking about I'm going to throw this one to you. When is congenital heart disease generally diagnosed in children?

So I speak to many patients that we will end up operating on in the neonatal period during fetal life basically. I talk to their parents. And the reason is we've gotten incredibly good at diagnosing congenital heart disease during fetal life. And particularly during the second trimester, somewhere between 18 and 24 weeks, we have an accuracy of diagnosis that's over 70%.

And so we can really talk specifically about what is the anticipated course for that particular child, unborn, very early on. So it serves the function of having the parents meet the team already and become familiar with who we are and what we can deliver for their children but also it gets them prepared mentally what is ahead.

So we can actually connect, for example, a mother whose fetus is diagnosed with transposition of the great arteries with another parent whose child was treated for that same condition. So right now, we have a overall-- if you take patients with congenital heart disease-- about 15% are diagnosed prenatally. There's a big chunk of patients, unfortunately, that is not diagnosed. And this has to do with access to health care and social stratification and geographical origin of the patient. But having said that, we do diagnose a lot of disease prenatally and with striking accuracy.

Great. Dr. Earing, I'm going to go back to you for a moment, because we've got a little bit of a technical issue that we want to take care of with Dr. Vricella. And it's very complex, Dr. Vricella. We're going to ask you to lower your blinds behind you so we can see your face.

Oh. OK.

Sorry. Dr. Earing, let's talk a little bit about-- just let's pick up on our earlier conversation and talk about the different services that are provided within the Heart Institute.

Yeah. No, I'm very fortunate. I have a tremendous team that helps Dr. Vricella and myself. And as we've developed this Heart Institute, we really want to look at all aspects of care.

So we have a fetal program that works with our maternal fetal medicine and the high-risk OBs all throughout the state to make those diagnoses that Dr. Vricella was talking about. But more importantly, if we have a diagnosis, how can that family deliver? Where should they deliver, and what's safest?

We have a group of individuals that are experts in electrophysiology or abnormal rhythms of the heart. Those people are really, really important to us. But we also are developing other programs.

After successful surgery, Dr. Vricella and Dr. Elzein and Dr. Hibino, after those children are born, they do really well. But often, because of the way they start their life, they need that little extra help. And so we have a neurodevelopment program and a high-risk program that follows them during those different stages.

Those are huge, huge aspects of what we do. We also have programs that look at Marfan syndrome, aortic aneurysm, and we could talk a little bit about that in the future. But adult congenital heart disease, if you have a patient that's born as a child, where do they get care as they get older? And those are things we talk about.

But we have a group of [? individuals with ?] pulmonary hypertension, which refers to pressures in the lungs being high. But sometimes after people having heart surgery, that can be a problem. We have a group of individuals specialize in that.

We also have diagnostic imaging, high-quality diagnostic imaging, advanced imaging. But more importantly, we can offer that now at all the different sites throughout our institutions. So people can have that locally, which is a huge, huge deal, Tim.

I'm very blessed to be able to work with such a great group of cardiologists, and we continue to develop these programs to actually help offset the needs of our growing population. It's a great thing.

And what we didn't talk about, Tim, is any child born today who has a heart defect, no matter how complex it is, they have 90% chance of surviving to be an adult. And that's a huge success story, and that is really why this population continues to grow. And we continue to develop our systems and meet the needs of those kids and now the adults as well.

Now touching upon something that you just talked about a moment ago, that care closer to home-- because, again, if you're a parent and your child is having a challenge like this, it's nice to not have to travel 200 miles or 1,000 miles or even 50 miles to get your care. And so I know that's a lot of the beauty of the CCHA and your work.

And I imagine you get a lot of road time with your job. Can you talk a little bit about that? And how does this work? Do you travel from facility to facility?

Yeah. No, what our goal is is we're developing teams of individuals that'll be primary in different areas. But the subspecialist, such as adult congenital, Marfans, EP, they're a smaller group. And we travel to the sites and offer those care locally.

And so it's a great, great opportunity to be able to do that and offer that care. Yes, I do drive a lot. I do have clinics. But this is more importantly is that we now have clinics-- we have clinics up north, west, south. We have them in Indiana.

And really, we have a site close enough to almost every place in the region. So it's just a really, really great opportunity to be able to provide that care home. I'm a dad. I have four children. I don't forget what it was like to try to get them in a car and drive them somewhere and get there on time.

It's a big deal, and I get it. That care closer to home was huge for me when my children were growing up, and it's something we take very seriously. And it's the right thing to do. It really is.

That's great.

To add to that, Tim, we were talking about fetal diagnosis. And this can be sometimes just a very emotionally challenging time for parents when they get the diagnosis. So our goal is to be immediately available to these parents.

So what I tell parents and colleagues is that you'll be able to get a hold of me or my colleagues by the end of the day, so you can really talk to us. And of course, now all these media platforms that we're utilizing for remote visits are helping us getting in connection with the patients right away.

Well, Dr. Vricella, apparently it's working. Because we have a comment on Facebook aimed at you. And it says, so grateful to have Dr. Luca in Chicago. Our son became a Heart Warrior because of him and his team. So I love seeing comments like that. I think that's

And they did get it right. It's Dr. Luca, as you can see here.

Dr. Luca. Yeah. I like that. So Dr. Vricella, or Dr. Luca, is there a way that parents can learn about the outcomes at different centers? Because I think a lot of people do like to comparison shop. And particularly, you do like to compare when you're talking about something that is this serious.

Yes. So we really believe in transparency. This is something that we really owe to our parents and our children. So transparency in this day and age is of paramount importance.

We have public reporting. So the data that we have about our outcomes is available online through The Society of Thoracic Surgeons. The group at Advocate and the University of Chicago has been really recognized for years with stellar outcomes.

So there used to be one, two, or three stars in terms of outcomes just to compare how we do as opposed to the remainder of the country. And the Advocate Health Care system has had three stars for years. So it's one way to really be able to look into the outcomes well and understand that specific center, what kind of outcomes they have. How do we compare to the rest of the country? So all that is readily available.

Again, for your child, it matters. It's more like a-- how can I say? You're looking for the best outcome possible and the center that will be ready to deliver that to you. And really, public reporting is meant to have available to everyone.

That makes sense. So Dr. Earing, we were talking a little bit at the beginning of the show, just mentioned it, about adults with congenital heart issues. And can you talk to us a little bit about that program?

And how do adults find out that they have these issues? Do they know, generally, from childhood? Or is that something that's sometimes found later in life?

Yeah, no, so Tim, I think that's a great question. About 20% of all adult congenital heart patients are patients that are newly diagnosed when they're adults. So they have a lesion or a heart defect that goes undiagnosed until they show up in adulthood.

So that's a small percentage, and examples of this being an abnormal aortic valve or a hole in the top chambers of the heart called an atrial septal defect. Those are the more common ones that we see.

But 80% of them that we see in the adult congenital program are really patients who have had heart surgery or have a genetic problem that was diagnosed when they were young. And as they grow up, with 90% of them now surviving to adulthood, we have this group of patients now that have really exploded.

So there's over a million adults with congenital heart disease living in the United States. And so this has created a group of individuals that have interesting lives, meaning that they all do well. They work. They have families just like all of us do.

But sometimes there are some complications late. Most of them do well. But it's really, really important that they get regular follow-up in a center experience with adults with congenital heart disease. And one of the problems then that people don't know is adult cardiology, they have about a month of training in congenital heart disease in their whole training.

Pediatric cardiologists really know congenital heart disease. But they're not trained in the adult type of things-- so high blood pressure, pregnancy, heart failure. Some of these are other things that they don't have that expertise.

The adult congenital program bridges that gap. And so with this huge group of patients that are growing, there's a huge need. The problem is there's only about 100 adult congenital trained physicians in the country.

And in Chicago, the really adult congenital programs really have been very small. We have a goal here of really, with this alliance, is we're trying to serve the adult congenital population for the first time in Chicago in a giant, giant system. And that's really exciting.

What you probably didn't know is that I used to be in Wisconsin. I ran the adult congenital program at the children hospital in Wisconsin. And that's a state about seven million people. We were seeing around 3,000 adult congenital patients a year.

Now picture a city like Chicago with 2.8 million and then another surrounding three million. The number of patients are staggering. And right now, there really is very, very few centers that are in Chicago at all to handle that care.

So we're here to change that. And I really, really am excited about that opportunity. I get really passionate and exciting about what I do. But again, I'm a pediatric and an adult-trained physician. And I love what I do every day, and I'm excited to come to work. I'm excited to do these things.

It's really interesting. And I would imagine, if you see some of the cases from your pediatrics patients and then you see some probably similar situations with some of your adult patients as well, it probably helps with your ability to treat these folks. We do have a question from a viewer. I'm kind of curious about this one as well.

When you have an adult with congenital heart disease, how common-- and I guess this could be a question for peds as well-- how common is it for somebody like that to need a heart transplant? Do you see that very often?

Yeah, no, that's great. So Dr. Vricella and our surgical partners are outstanding. But when you're born with an abnormal heart-- and we palliate them with different procedures. They really do well for a long time.

But sometimes those ventricles or those valves that we fixed, they wear out. And some patients do need to have a heart transplant. But more importantly, they need to have sometimes medical therapy to help them get through this.

So transplant, as the adult congenital population is getting older, we're seeing more and more heart failure and more and more needs for transplant. But the numbers still are small. We have some great, great outcomes. But it is something that's a growing problem.

We are partnering with groups at NorthShore, partner with the adult groups at University of Chicago. We partner with the groups at Advocate. And really, we're really developing really complex systems to help take care of the patients within all those systems.

So I think it's a great question, Tim. And that's really one of the things that we're really trying to develop here as well. So I'm very excited about that, because I know we can meet those needs. Luckily, the numbers are still small. But it is a growing population as they get older.

So, Dr.-- go ahead.

Just to add to what Mike was saying, these cases, we're going to be seeing more and more of these. And they're very challenging. So they really need the tertiary level center to take care of these patients.

Because from a technical standpoint, you have to put a normal heart into an abnormal recipient, in terms of how they're structure is. So these cases, they're all re-operative cases. Some of them will be bridged with a mechanical assist device to help the patient being bridged to transplant. So these cases are very challenging and are really quintessential multidisciplinary efforts. But we're going to be seeing more and more of these.

That's great. So this is a question from a viewer. This is from Megan. And the question is, does the CCHA work together to put the babies and kids with congenital heart disease with a facility or surgeons that are best suited for their specific defect? How does that work? How do you decide what patient is seen where? I guess that's the question. And Dr. Earing, I don't know if you want to take that one or Dr. Vricella.

[INAUDIBLE] Sorry. [INAUDIBLE] Again, we really start with care closer to home. We try to make sure that the cardiologists are matched with the families.

Then we work as a team to decide where those surgeries are, depending on what the age and what they're looking for. Dr. Vricella, Dr. Elzein, Dr. Hibino, they're outstanding. And I'm really fortunate because not only that, they work so well together. it just makes a very great team.

So when we get a diagnosis from fetal life, we look at those. And then we really work with the families and the maternal field medicine where it's safe for them to deliver, whether they deliver locally. Or do they need to deliver at the hospital where they're going to have surgery?

That's just a really, really important aspect, because some children need to have intervention immediately after birth. And those are the ones that we would deliver more at one of our tertiary hospitals where we have all our team ready to go with that. Vast majority, though, we try to deliver locally if we can.

Fantastic.

Yeah, I think we do stratify it by the disease. And as Mike was saying, we do operate in two main campuses-- University of Chicago and Advocate Children's Hospital. But the patients come in through many different portals.

And so we really have to tailor their hospital course according to the specific disease. So some children will come down to Advocate to get delivered over there, some at University of Chicago, some at Lutheran General, some at NorthShore.

We have to design the perfect suit for the baby. And so there's no two suits that are exactly the same. And for that one time where you're going to have a major operation as a newborn, you want to go where the care delivered really matches the severity of the disease of your child.

Yeah, that makes sense. So Dr. Vricella, question about heart failure in pediatric patients. How common is that, and what are some of the treatments that you can perform for children that issue?

Yes, and so we're definitely seeing more and more patients with heart failure. You could say, generally, that 20% of patients with heart disease will actually encounter heart failure at some point in their preoperative course. And the reason is if you have structural heart disease, it translates into heart failure, which is the inability to meet the demands of the body in terms of pumping oxygen and blood around-- seeing in a very simplistic way.

But the patients who have structural heart disease that can be corrected or palliated get out of heart failure. There is, however, a large population of patients who actually have structure heart disease that has been corrected that, for some reason, like Dr. Earing was saying, their hearts are not good anymore in terms of their pumping capacity. Basically there's a valve or dysfunction, or they've been repaired many, many years prior. And the heart function is just getting worse.

And then there's children and young adults in whom the muscle actually will fail, so patients with myocarditis or dilated cardiomyopathy. Those patients all will need to be channeled into a system that takes care of their heart failure. And treatment of heart failure is sort of a step-wise approach.

You start with medical management of heart failure with medications, with various forms of support. Then you can go into mechanical support of the failing heart and eventually heart transplantation, which is the ultimate goal. If you have a heart that is, of course, failing, is beyond being repaired or palliated, your ultimate dream form of therapy is cardiac replacement with a heart transplant. So it's important to have a team that actually understands and implements all these different forms of treatment for heart failure, from medical management to heart transplantation.

So what does the Heart Institute-- I mean, specifically, you were telling about that team effort. But as you work with the families and the children-- I guess this goes back to Dr. Earing's answer earlier-- you really tailor that treatment to that specific child. And it depends on the condition where they will receive that treatment.

It's got to be kind of a balancing act. But apparently, you all perform this very well. Do you all meet and discuss this? How does that work?

Tim, no, that's great. As a group, we have our entire alliance meets on Wednesday. And we talk about every surgical case. We also talk about a complex disease about what we should do with each patient, et cetera, Vricella and our surgeons, Dr. Elzein and Dr. Hibino.

But it's all the cardiologists. It's the nurse practitioners. It's our critical care doctors, so the doctors in the ICU that take care of our patients, anesthesia. They're all there. So that conference is just an incredible conference where we share ideas, we learn together, and then we work together about what kind of a plan would be best for that patient and that family.

Great. Another question from a viewer, and this one deals with COVID. Is there any new knowledge on the impact that COVID might have on children with congenital heart disease?

Yeah. So I think everything is moving fast, and everything continues to change. We do know that COVID can have an impact on children as well as adults. In the adult congenital world, we know that there are certain patients that have single ventricles or have high pressures in their lungs. They've struggled with COVID, and they've had more issues than those patients that have more simple [? presentations. ?]

In the pediatric population, we've found similar findings. But in general, the pediatric population have not had as many issues as our adult congenital patients that we've seen. So it's a great, great question. We're really following our patients very closely, and I think, again, we're really trying to keep everybody safe as best we can.

We don't have a lot--

Yeah, I know, and--

Go ahead. I'm sorry.

Just to add to that, this is a question I get from a lot of parents as they're either approaching surgery and about timing of surgery. How do we deal with COVID? And that, vis-á-vis our child who needs an operation, as the child approaches the hospital, what do we do?

And I can tell you that the precautions that has been put in place have worked very, very well. And I don't think that has to have an impact on the decision to proceed with surgery or being overly worried about going to the hospital to get cardiac care. We've been doing so many cases over this pandemic, and we didn't have one patient turned positive during their hospital stay. So I would just like to say to our viewers that it's OK. I mean, if your child needs heart care in the era of COVID, we can deliver that.

Yeah, that's one thing that we want to stress, and we do this often on the show. If you do need medical care, please don't avoid medical care because of COVID. It's very important to get things taken care of. These hospitals are very safe places to be, and your teams take extraordinary care to make sure that the patients and their families are very safe when they come for the care.

I just can't stress that enough. I think very important. We're about out of time, but there's a couple of questions I want to get to real quickly before we wrap this up. And Dr. Vricella, going to have to be a short answer if you can. Talk to us just what is an aortic aneurysm, and how often does that need to be repaired?

Yeah, so an aortic aneurysm is the enlargement of the aorta to a point at which it becomes concerning for the possibility of rupture or dissection, which is basically a catastrophe.

So in terms of repair, we want to do this prophylactically. We want to get to someone's aorta before a catastrophe can happen. So we have guidelines. We follow these patients.

These patients can be patients with congenital heart disease or connective tissue disorders. This is one of the things we also specialize in and that we want to develop across the alliance.

And Dr. Vricella, or Dr. Earing-- either one of you can answer that-- if people want to learn more about the Heart Institute and the CCHA, what's a good way to do that?

Great. No, the Comer Children's website, as well as Advocate Children's website has the Heart Institute listed on it. You can link on those really quickly. You can find all our specialty programs as well.

Dr. Vricella and myself are open to any questions, and our emails are on there. We'd be happy to discuss anything that the audience had. We're really, really, really excited to be here. I've been here for six months, and I really have appreciated the opportunity to be here.

And I'm a Chicago guy, and I'm really happy that we're able to do this together. And we look forward to working with all the families and keeping your children safe.

And I know--

Yeah, and--

Oh, go ahead, Dr. Vricella. I'm sorry.

I just wanted to reaffirm, if you want to reach out, we'll get back to you by the end of the day.

That's fantastic. And I do know that CCHA is-- we're building out a website-- or we're not, but one is being built out. I'm not building a website. But one is happening, so that'll happen in the near future as well. I know we're going to do more of CCHA programs in the future.

You guys were fantastic. I appreciate you taking time out of your day. I know you're very busy. I know Dr. Vricella just came from a case, in fact. And Dr. Earing, you're in clinic today. So we really appreciate you doing this.

Delighted to be here.

Also, thank you to our viewers. Those of you who've watched and participated in the program today, great questions, as always. Please remember to check out our Facebook page for our schedule of programs that are coming up in the future.

If you want to make an appointment online, you can go to uchicagomedicine.org or call us at 888-824-0200. That's for UChicago. We also have the-- I think we have the-- what's the other lower-third, John, if you can put that up for Advocate? I'm sorry. I'm throwing this at you.

There we go, because we want to give them credit as well. CCHA is a very important thing, and it's a team effort. Thanks again for being with us today, and hope everybody has a great weekend.

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